Ocular-central nervous system lymphoma mimicking posterior scleritis with exudative retinal detachment

Objective: We describe an unusual ocular presentation of ocular-central nervous system lymphoma in a young patient. Design: Interventional case report and literature review. Methods: A previously well 24-year-old white woman presented with left eye pain and reduced vision. Episcleral injection, globe tenderness, an afferent pupil defect, and exudative retinal detachment were present. Computed tomographic scan of the head and orbits demonstrated scleral thickening, retinal detachment, and no other abnormality. A provisional diagnosis of posterior scleritis with exudative retinal detachment was made. Investigation for underlying connective tissue diseases was negative. There was an initial prompt response to corticosteroid therapy. The patient's symptoms and signs then recurred, and a left third cranial nerve palsy developed. Systemic investigations including lumbar puncture ultimately led to the diagnosis of primary T-cell central nervous system (CNS) lymphoma. Serologic tests for human immunodeficiency virus were negative. Main Outcome Measures and Results: The patient underwent orbital and cranial irradiation and intrathecal and systemic chemotherapy, Despite an initial response to treatment, she returned with a recurrence of the lymphoma in the anterior segment of the left eye. Her systemic disease progressed rapidly, and she died shortly thereafter. Conclusions: This patient's young age and initial presentation mimicking posterior scleritis with unilateral exudative retinal detachment, without evidence of vitreous involvement, are highly unusual for ocular involvement in primary CNS lymphoma. A review of the literature highlights the atypical nature of this presentation. Ophthalmology 2000; 107:1955-1959 (C) 2000 by the American Academy of Ophthalmology.